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PubMed2000

HELLP syndrome.

HELLP syndrome is a serious, life-threatening form of pre-eclampsia with a typical laboratory triad. The incidence of the disease is reported as being 0.17-0.85% of all live births. There has been, to date, neither reliable early recognition nor effective prevention of HELLP syndrome. As a result of endothelial dysfunction, activation of intravascular coagulation occurs with fibrin deposition in the capillaries and consecutive microcirculation disorders. The disease manifests itself on average between 32-34 weeks' gestation. HELLP syndrome will occur postpartum in up to 30% of the cases. The clinical cardinal symptom of the disease is right upper quadrant pain or epigastric pain accompanied with nausea, vomiting and malaise. In 20% of the cases with HELLP syndrome there is no hypertension and 5-15% of the pregnant patients present a low level of proteinuria or none at all. The early recognition of hemolysis is most sensitively managed by the determination of the serum haptoglobin. The increase of the aspartate transaminase (AST) and the alanine transaminase (ALT) often precedes a decrease in platelets. The course of HELLP syndrome is incalculable. It is universally agreed that a pregnancy from 32-34 weeks should be immediately delivered. Before 32-34 weeks, expectant management is generally possible in a perinatal center. The frequency for a repeated hypertensive disease in pregnancy ranges from 27% to 48%.

Diagnosis, Differential↗
PubMed2005

General anesthesia with remifentanil for Cesarean section in a patient with HELLP syndrome.

HELLP syndrome is a severe complication of pre-eclampsia characterised by hemolysis, elevated liver enzymes and a low platelet count. It is associated with an increased risk of adverse outcome for both the mother and the fetus. Patients with HELLP syndrome are also at greater risk of pulmonary edema, adult respiratory distress syndrome, abruptio placentae, intracerebral hemorrhage, eclamptic convulsions, disseminated intravascular coagulation, ruptured liver hematomas and acute renal failure. Perinatal mortality is equally high. Before delivery, aggressive obstetric management is directed toward stabilization of the affected organ systems, if possible, and interruption of the pregnancy in the early phase of the accelerated disease progression. Definitive therapy is delivery. Parturients HELLP syndrome often require general anesthesia for Cesarean section delivery. The anesthetic technique is critical for these patients with a high risk of uncontrollable hypertension, bleeding and multiple organ failure. Remifentanil is increasingly used as a very short analgesic agent providing cardiovascular stability in high-risk patients. We report the management of a patient presenting in labor with HELLP syndrome, and describe the successful use of remifentanil as part of the anesthetic technique for her subsequent Cesarean section.

Adult↗
PubMed2004

[Newborn of a mother with HELLP syndrome].

HELLP syndrome is a serious complication of pregnancy with characteristic appearances between 22 to 26 weeks of gestation. Clinical classification in three groups, is based on platelet count. Early diagnosis and appropriate management allow women to achieve a mortality rate below 1%. Neonatal complications are strongly associated with a newborn's immaturity and class of HELLP syndrome. In both presented cases despite identical maternal treatment (steroids because of I st class of HELLP syndrome) neonatal clinical courses were different due to the differences in gestational age. However, appropriate prenatal and neonatal care in tertiary centers was successful.

Adult↗
PubMed1993

Type 1 diabetes mellitus developing during HELLP syndrome.

HELLP syndrome, a combination of haemolysis, elevated liver function tests and a low platelet count, is described as a rare but serious complication of pregnancy. Some authors term HELLP syndrome an "acute autoimmune status". This thesis is based upon positive platelet IgG antibodies, autoimmune haemolysis and passive disease transfer to the fetus. We diagnosed the onset of type 1 (insulin-dependent) diabetes mellitus in a 30-year-old patient with HELLP syndrome during the 35th week of pregnancy. As immune mechanisms, together with other factors, play a fundamental role in the development of type 1 diabetes, a combination of autoimmune reactions could explain the onset of type 1 diabetes in a patient with altered immune status. To our knowledge this is the first report of such an association.

Adult↗
PubMed2004

[Pregnancy complicated by HELLP syndrome].

HELLP syndrome belongs to the group of pathological disorders associated with pregnancy-induced hypertension and may accompany preeclampsia. The basic criteria for establishing the diagnosis are as follows: H--for hemolysis, EL--for elevated liver enzymes and LP--for low platelets. In this report the authors present the case of a 32 years old primipara admitted to the Obstetrics Clinic complaining of epigastric pain, nausea and vomiting. Medical history revealed previously diagnosed and treated reflux disease. In the 39th week of gestation epigastric pain manifested again, blood pressure was high (150/100) and on the basis of laboratory parameters HELLP syndrome was diagnosed (GPT 319 U/L, GOT 204 U/L, platelet 80 x 10(9)/L, antithrombin III 63.9%, D-dimer (+++)). With this case report, authors wished to point out the importance of early diagnosis and treatment of this rare, but having high percentage of perinatal mortality syndrome.

Adult↗
PubMed1988

[Significance of early laboratory diagnosis for obstetrical procedures in severe gestoses and the HELLP syndrome].

HELLP syndrome is a severe complication of preeclampsia, incalculable in its course and involving high risk for mother and fetus. Over a period of three years and eight months a total of 24 patients with HELLP syndrome were treated at Göttingen University Gynecological Clinic and the Gynecological Clinic of Munich Technical University. In all cases the correct diagnosis had already been made by the referring physician or by the obstetrician on admission, taking laboratory parameters into account. The chief clinical symptom was upper abdominal pain, All of the patients presented with thrombocytopenia and elevated liver enzymes. The signs of hemolysis derived from the increased bilirubin concentrations and the LDH; in 13 cases fragmentocytes were detected in the peripheral blood smear. No indications of a pronounced DIG were found. The mean time elapsed between hospitalization and delivery was six hours (0.5-40 hours); 22 patients were delivered within 24 hours. Cesarean section was performed in all cases. The mean gestational age at the time of birth was 35 weeks (25-38). Three of 24 children died prepartially; with the surviving infants no major problems arose in the postnatal course. In 19 cases the abdominal cesarean delivery and clinical course were without complications. Reoperation, in two cases combined with a puerperal hysterectomy, was necessary in three patients, due to subfascial and intraperitoneal or retroperitoneal hematomas.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗
PubMed1999

A review of HELLP syndrome.

HELLP (hemolysis, elevated liver enzymes, and low platelets) syndrome is a variant of severe preeclampsia which is associated with substantial maternal and perinatal morbidity and mortality. As with preeclampsia, the etiology and pathogenesis of HELLP syndrome is not completely understood. An increase in vascular thrombosis and activation of the coagulation system may be important in the clinical presentation of this disorder. Laboratory criteria for the diagnosis of HELLP syndrome have been classically described but lack uniformity among different institutions. Aggressive management of HELLP syndrome with expeditious delivery appears to yield the lowest perinatal mortality rates. Conservative or expectant management has been associated with higher stillbirth rates with antenatal corticosteroids not causing resolution of the laboratory abnormalities. Resolution of laboratory abnormalities in HELLP syndrome runs a protracted course over several days after delivery. Despite nearly two decades since HELLP syndrome has been defined as a clinical entity, treatment for the disorder still remains delivery of the patient.

Delivery, Obstetric↗
PubMedDate not supplied

[Ruptured subcapsular hepatic hematoma associated with HELLP syndrome].

HELLP syndrome is a rare condition related to pregnancy; however, it can cause complications such as a ruptured subcapsular hepatic hematoma. In most cases, this is managed surgically, but the mortality rate is high, up to 50% of all patients. We present a case with conservative management by percutaneous embolization of the right hepatic artery with a successful outcome and we propose a modern treatment algorithm.

Adult↗
PubMed1992

[Massive proteinuria and HELLP syndrome].

HELLP syndrome continues to be a clinical entity of difficult diagnosis. Weinstein first defined it in 1982 giving the practicing obstetrician a sequence of useful initials (H = hemolysis; EL = elevated liver enzymes; LP = low platelets). Since then a lot has been written and it has become clear that the syndrome is a form of severe preeclampsia. The American College of Obstetrics and Gynecology does not include HELLP in the description of severe pre-eclampsia as such but does accept each of its components as being part of severe pre-eclampsia. The case presented deals with a 33 year old white female, admitted at 27 weeks gestation with nausea, epigastric pain resembling acute abdomen, nose bleeding and mild hypertension. The analysis revealed an abnormal liver profile with elevated GOT, GPT and LDH, heavy proteinuria (14.4 g/day), decreased platelet count (92000/mm3) and elevated total bilirubin. Pregnancy was terminated by cesarean section 24 hours after admission because the patient's condition was deteriorating. Obviously in pre-eclampsia/eclampsia there is a systematic injury to all tissues. Proof of this is the hypertension as a consequence of vascular spasm and proteinuria due to glomerular injury. In HELLP the sequence of events is probably altered; hepatic injury precedes vascular and renal injury of conventional preeclampsia. The syndrome results from many clinical and pathological symptoms derived from endothelial microvascular injury which determine a rapid platelet activation causing vascular spasm, platelet aggregation and further endothelial injury through a feedback mechanism.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗
PubMed1987

HELLP syndrome.

HELLP syndrome is a triad of hemolysis, elevated liver enzymes and low platelet count during pregnancy and it is proposed to be a sign of severe preeclampsia. We present two mothers with this life-threatening condition. In the first case, the syndrome appeared after a twin delivery at 34 weeks of pregnancy. The mother required 10 days of intensive care with blood and thrombocyte transfusions. Both she and the infants survived. In the second case, the mother had all classic signs of severe preeclampsia at the 27 week of pregnancy. After 3 days' intensive care, a cesarean section was performed and both the mother and the child survived.

Adult↗
PubMed2003

[Primary antiphospholipid syndrome associated with HELLP syndrome in pregnancy].

Authors report the case of a pregnant woman with antiphospholipid syndrome associated with HELLP syndrome. Pregnancy was terminated because of severe preeclampsia. However the patient's condition worsened. New symptoms of antiphospholipid syndrome developed (deep vein thrombosis, ischaemic optic lesion) and ARDS, therefore respiratory therapy was introduced. Plasmaphereses were performed concomitantly with high-dose intravenous immunoglobulin, glucocorticoid, cyclophosphamide and anticoagulant therapy. She responded to the therapy well, and eventually recovered completely. There are only a few case reports about pregnancies complicated with the association of antiphospholipid syndrome and HELLP syndrome. To authors' best knowledge, this is the first Hungarian report about this rare and curious association and it's successful treatment.

Adult↗
PubMed2005

[31-year-old gravid woman of reversible posterior leukoencephalopathy syndrome associated with HELLP syndrome after labor].

We report on a 31-year-old gravid woman with reversible posterior leukoencephalopathy syndrome (RPLS) associated with HELLP syndrome. The patient was hospitalized and underwent an emergency cesarean section at the 34th week of pregnancy due to severe toxemia. After labor her blood pressure acutely increased to 180/100 mmHg and a generalized convulsion occurred. Laboratory data revealed hemolysis, elevated liver enzymes, and low platelets, consistent with HELLP syndrome. FLAIR and Apparent Diffusion Coefficient images on MRI showed hyperintense lesions bilaterally in the posterior white matter, however Diffusion-Weighted images revealed slightly hyperintense changes in the same locations. With the use of diltiazem for blood pressure control, and anticonvulsants, convulsions disappeared and consciousness level improved. Moreover the MRI abnormalities also improved after therapy. This case was diagnosed as RPLS associated with HELLP syndrome and is the first case of an investigation utilizing Diffusion-Weighted and Apparent Diffusion Coefficient images to establish this diagnosis. Based on these results, possible mechanisms of RPLS may be vasogenic edema mediated by a cerebrovascular endothelial disturbance of cerebral vessels and a rapid blood-pressure increase due to HELLP syndrome.

Adult↗
PubMed2004

Thrombotic microangiopathy with liver, gut, and bone infarction (catastrophic antiphospholipid syndrome) associated with HELLP syndrome.

Hemolysis, elevated liver enzymes, low platelet count (HELLP) syndrome is a thrombotic microangiopathy complicating pregnancy and shares many clinical and biological features with thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS). Thrombotic microangiopathy is also a pathological feature of catastrophic antiphospholipid syndrome (CAPS). An association between refractory HELLP syndrome and antiphospholipid syndrome (APS) has been reported in a few cases. We describe a 19-year-old woman with APS and multiorgan failure conforming to a diagnosis of CAPS who developed refractory HELLP syndrome.

Adolescent↗
PubMed1998

Maternal and perinatal outcome after expectant management of the HELLP syndrome compared with pre-eclampsia without HELLP syndrome.

OBJECTIVE: To compare maternal and perinatal outcome of pregnancies complicated by pregnancy induced hypertension and HELLP syndrome with the outcome of pregnancies complicated by pre-eclampsia only. DESIGN: It was a retrospective cohort study. Fifty one patients with pregnancy induced hypertension and HELLP syndrome were matched with 51 pre-eclamptic patients according to parity and gestational age on admission in hospital. Management was expectant, treatment only symptomatic and delivery was mainly effectuated because of fetal condition. RESULTS: There was no maternal mortality in either group; maternal morbidity was more frequent in the HELLP group. Immediate intervention within a few hours of admission because of fetal distress more often occurred in the HELLP group. In both groups 41 children (80%) are still alive, with one major handicapped child in each group. Logistic regression analysis identified gestational age on admission and antihypertensive treatment on admission as significant contributors to perinatal mortality or major handicap. Whether the patient belonged to the HELLP group or the pre-eclamptic group had no influence on outcome. CONCLUSION: Expectant management of pregnancy induced hypertension with HELLP syndrome and pre-eclampsia without HELLP syndrome results in similar maternal and perinatal outcome. Perinatal outcome is strongly influenced by gestational age and the severity of hypertension as expressed by the need of antihypertensive treatment, irrespective of the underlying syndrome.

Adolescent↗
PubMed1995

Coagulation and plasma fibronectin parameters in HELLP syndrome.

OBJECTIVES: HELLP syndrome (hemolysis, elevated liver enzymes and low platelets) has a high fetal mortality and maternal morbidity, partly due to its late diagnosis. In order to facilitate earlier diagnosis, we studied the changes occurring in natural coagulation inhibitors, fibronectin and haptoglobin as potential early markers of endothelial damage, coagulation cascade activation and intravascular hemolysis. METHODS: The study compared antithrombin (AT-III), protein C and S activity, plasma fibronectin, 'prothrombin time' and 'partial prothrombin time' (AST, ALT), lactate dehydrogenase (LDH), bilirubin and serum haptoglobin in 17 asymptomatic controls, 19 preeclampsia patients and 11 HELLP syndrome patients. RESULTS: HELLP syndrome patients had higher fibronectin and D-dimer values, lower AT-III and protein C activity, a lower platelet count and higher LDH than healthy controls; only 25% had raised bilirubin. Serum haptoglobin was lower in HELLP syndrome. CONCLUSIONS: Early on in HELLP syndrome, there is probably a pro-coagulatory imbalance in the placental microcirculation. Endothelial damage causes tissue thromboplastin release and coagulation cascade activation due to collagen exposure; the vascular lesion increases thromboplastin in the bloodstream and triggers distant coagulation processes, suggesting compensated disseminated intravascular coagulopathy. Measuring plasma fibronectin and coagulation inhibitors should be supported by testing haptoglobin as a marker of intravessel hemolysis to differentiate conventional preeclampsia from HELLP.

Adult↗
PubMed2006

[The rate of maternal complications in 107 pregnancies complicated with HELLP syndrome].

INTRODUCTION: HELLP syndrome (Haemolysis, Elevated Liver enzymes, Low platelet count) is a grave, life threatening form of preeclampsia, which was named by Weinstein in 1982, on the basis of characteristic changes in laboratory findings (haemolysis, elevated level of liver enzymes and thrombocytopenia). OBJECTIVE OF THE STUDY: To assess the rate of maternal complications in HELLP syndrome. MATERIAL AND METHODS: In the past ten years, 107 patients were treated for HELLP syndrome at the Intensive Care Unit (ICU) of the 1st Department of Obstetrics and Gynaecology, Semmelweis University. The authors summed up about their experience with the treatment of patients, with special regard to the typical symptoms of HELLP syndrome, the course of the disease, postpartum maternal complications. RESULTS: The frequency of HELLP syndrome in live births was found to be 0.37%. In 96% of the patients the pregnancy was terminated via Caesarean section. Pulmonary oedema was the most common cardiopulmonary complication (11%) and developed in Mississippi Group I in the majority of the cases (21%). Transfusions had to be given quite frequently; 62% of the patients in the study were transfused using erythrocyte preparations. In persistent or progressive cases in the postpartum period, the elimination (uterine curettage and lavage) of factors responsible for the persistence of the disease (toxic and vasoactive agents in the endometrium) resulted in the recovery of one third of the patients. Maternal thromboembolic complications developed in 11% of the patients, each of them was affected in Mississippi Group I, with the lowest platelet count. CONCLUSIONS: The immediate termination of a pregnancy in which HELLP syndrome emerges may save the patient's life. It is recommended to try and lift foci applying uterine curettage and lavage as the first step, if the mothers' condition persists or progresses after delivery. Its development is accompanied by a significant increase in maternal and fetal morbidity and mortality alike, therefore it is essential for the obstetricians to be familiar with the disease.

Adult↗
PubMedDate not supplied

[Diagnostic and therapeutic problems in HELLP syndrome].

The HELLP syndrome is a severe and life-threatening complication of preeclampsia. Despite obstetricians are more aware of this syndrome maternal and perinatal mortality could not be reduced during the last years. The diagnostic problems of the HELLP syndrome are early detection of the disease, problems concerning differential diagnosis to other diseases with similar symptoms, and the different definition of the HELLP syndrome itself. For early detection of hemolysis determination of serum haptoglobin should be included in laboratory screening methods. Manifestation of DIG is not a principal sign of the HELLP syndrome but reflects a secondary pathophysiological process of the primary disease. Therefore administration of heparin and antithrombin III is not indicated and may lead to induction or amplification of life-threatening bleedings in these cases. Conservative approaches have shown to improve the fetal and maternal condition in individual cases, however, termination of pregnancy is the only definitive cure for patients with HELLP-syndrome. With respect to our perinatal results (n = 84) immediate delivery, predominantly by caesarean section, is recommended after diagnosis has been confirmed.

Cesarean Section↗